Caring for Glut1 DS

There are currently no approved medicines for Glut1 DS, but there are treatments that may be helpful in managing symptoms. Talk to your doctor before making any diet or lifestyle changes.1

A ketogenic diet managed by a nutritionist is currently considered to be the main treatment option for this condition. It is a high fat, low or adequate carbohydrate, and low or adequate protein diet, which is designed to provide an alternate energy source—called ketone bodies—for the brain.2

Even if you’re on a ketogenic diet, be sure to talk to your health care provider and ask about:

Antiepileptic treatments

Things that may interfere with glucose transport (eg, caffeine and diazepam)3

Regular health checks with a Glut1 DS specialist

Connecting with other people for insights about coping with the disease

Antiepileptic drugs4

People with seizures are often treated with antiepileptic drugs (AEDs) to control the number and severity of seizures. However, when the underlying cause is Glut1 DS, the seizures may not respond to AEDs as expected. There are no AEDs approved specifically for people with Glut1 DS. Talk to your doctor about your specific questions relating to AEDs.

Ketogenic diet2,4

Today, the most common way to manage Glut1 DS is through a high fat, low or adequate carbohydrate, and low or adequate protein diet called the ketogenic diet (KD). When the body cannot use its primary fuel—glucose—it will use other sources of energy. One of these sources is from fat, which the body turns into an energy source called ketone bodies. Ketone bodies can bypass the faulty glucose transmitters in people with Glut1 DS.

For some, the ketogenic diet is effective in reducing seizures but compliance can be difficult. The effectiveness of the ketogenic diet in the treatment of developmental delays and movement disorders has not been confirmed.

A ketogenic diet needs to be designed by a nutritionist specifically for the needs of each patient. It must be monitored and may be modified, depending on the individual’s response. The classic ketogenic diet has 4 grams of fat to every 1 gram of carbohydrate and protein combined (4:1 ratio).

Some people are able to use a modified ketogenic diet (3:1 ratio) or even a modified Atkins diet (2:1 or 1:1 ratio), but there are many factors, including possible side effects, to be taken into consideration. All diets must be fully supplemented with vitamins and minerals. The diet plan should not be altered without talking to a health care professional.

Because following a ketogenic diet may be difficult for some, it can be helpful to go to a center that specializes in designing and monitoring ketogenic diets.